PURPOSE: To report atypical central serous chorioretinopathy and choroidal thickening in a patient with heritable pulmonary arterial hypertension. METHODS: A 40-year-old man with heritable pulmonary arterial hypertension presented with blurred vision in his left eye and was followed up for 1 year with clinical examination, enhanced depth optical coherence tomography, fluorescein and indocyanine green angiography, and fundus photography. RESULTS: At presentation, atypical central serous chorioretinopathy with multiple retinal pigment epithelial detachments, a thick subfoveal choroid, and dilated choroidal vessels were seen in the patient's symptomatic left eye. After treatment for pulmonary hypertension, the serous detachments disappeared and choroidal thickness gradually decreased over a period of 4 weeks and remained unchanged at 13 months of follow-up. CONCLUSION: Central serous chorioretinopathy and choroidal thickening that responded to treatment of pulmonary arterial hypertension suggest a pathophysiological link between pulmonary arterial hypertension and central serous chorioretinopathy, perhaps mediated by choroidal venous stasis.